OBJECTIVE: The aim of this study was to investigate clinical features and outcomes of children treated for congenital syphilis (CS).METHODS: Infants born alive in the public sector of São Lucas Hospital, Porto Alegre, Brazil, 1997 to 2004, whose mothers had syphilis and neonates with CS born in other facilities and admitted during this period were included. Follow-up was performed from birth up to 5 years.RESULTS: Among 24,920 live births, 379 (1.5%) met the criteria for CS. A further 19 infants born in other hospitals were included, for a total of 398 with CS. We compared infants with CS with 120 infants whose mothers received adequate treatment of syphilis before delivery (total sample, 518 infants). Congenital syphilis was associated with delivery before 34 weeks, low birth weight, and small for gestational age. During the study period, 37 stillbirths with CS were detected. Result from the serum venereal disease research laboratory test was negative at birth in 17.5% of the neonates with CS, and in 4 infants, it became positive after the second day. Thirty percent of the infants with CS were reevaluated between 8 and 60 months, and most had a good outcome when managed according to standard guidelines. Sixteen infants (13.3%) had sequelae. Of these, 8 were symptomatic in the neonatal period, and 13 (81%) of 16 had laboratory/x-ray findings. All asymptomatic and 78% of symptomatic infants had nonreactive fluorescent treponemal antibody absorption test after 12 months of age.CONCLUSIONS: Congenital syphilis remains an impacting disease that causes fetal and neonatal deaths, prematurity, low birth weight, and severe and irreversible sequelae in some children. This study confirms the value of standard guidelines for its management.